Childhood-onset Sjögren disease : clinical phenotype compared with adult-onset disease, disease course and treatment patterns in a nationwide Italian multicentre pediatric cohort

Childhood-onset Sjögren disease (cSjD) is a rare autoimmune disease with an incompletely characterized phenotype, limited longitudinal data, no validated classification criteria, and limited evidence to guide treatment. To compare the clinical and serological phenotype of childhood-onset and adult-onset Sjögren disease (SjD) and to describe disease course of the pediatric cohort. This cross-sectional study included children with cSjD fulfilling the SjD 2016 ACR/EULAR criteria recruited from 22 Italian pediatric rheumatology centres and adult-onset SjD patients from a tertiary referral centre. Demographic, clinical, laboratory, ESSDAI, clinESSDAI, ESSPRI, and treatment were compared. Longitudinal pediatric data were analysed descriptively, and factors associated with systemic treatment initiation were explored. The final cohorts included 102 cSjD and 140 adult patients. Children more frequently presented with recurrent parotid enlargement (44% vs. 13%, p < 0.0001), cutaneous involvement (25% vs. 8%, p < 0.0001), lymphadenopathy (24% vs. 9%, p = 0.001), arthritis (17.7% vs. 6.4%, p = 0.006), and fever (12% vs. 3%, p = 0.006), whereas sicca symptoms were significantly less common (xerostomia 45% vs. 94%; xerophthalmia 36% vs. 86%; both p < 0.0001). cSjD showed higher ESSDAI (median 5.5 vs. 1.0, p < 0.0001), higher serum IgG, but lower anti-SSA/anti-SSB positivity. During a 26.4-months median follow-up, 87.5% initiated systemic treatment. In exploratory analyses, higher baseline ESR was independently associated with a shorter time to systemic treatment initiation. Compared with adult-onset SjD, cSjD showed more frequent systemic inflammatory involvement and less prominent sicca manifestations. Pediatric-specific classification criteria, outcome measures, and prospective studies to optimise management and improve long-term outcomes are still crucial unmet needs.

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Journal
Arthritis Research & Therapy
Published
2026-10-27
DOI
https://doi.org/10.1186/s13075-026-03925-4
Primary Topic
Salivary Gland Disorders and Functions
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article
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article

Childhood-onset Sjögren disease : clinical phenotype compared with adult-onset disease, disease course and treatment patterns in a nationwide Italian multicentre pediatric cohort

Angela Pistorio, Adele Civino, Andrea Uva, Francesca Alessi et al.
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Arthritis Research & Therapy
Salivary Gland Disorders and Functions
article

Childhood-onset Sjögren disease : clinical phenotype compared with adult-onset disease, disease course and treatment patterns in a nationwide Italian multicentre pediatric cohort

Angela Pistorio, Adele Civino, Andrea Uva, Francesca Alessi, Gabriele Simonini, Maria Francesca Gicchino, Francesco Ciccia, Emanuela Del Giudice, Roberta Loconte, Clara Malattia, Silvana Ancona, Achille Marino, Alessandra Meneghel, Enrico Tirri, Roberta Naddei, Carlotta Covizzi, Valentina Ibba, Angela Mauro, Donato Rigante, Sara Pessano, Emma Longoni, Saviana Gandolfo, Francesco Baldo, Marco Gattorno, Flavia Riccio, Edoardo Marrani, Luciana Breda, Michela Cappella, Sofia D’Elios, Serena Pastore, Simona Campus, Valeria Manfrè
article en

Abstract

Childhood-onset Sjögren disease (cSjD) is a rare autoimmune disease with an incompletely characterized phenotype, limited longitudinal data, no validated classification criteria, and limited evidence to guide treatment. To compare the clinical and serological phenotype of childhood-onset and adult-onset Sjögren disease (SjD) and to describe disease course of the pediatric cohort. This cross-sectional study included children with cSjD fulfilling the SjD 2016 ACR/EULAR criteria recruited from 22 Italian pediatric rheumatology centres and adult-onset SjD patients from a tertiary referral centre. Demographic, clinical, laboratory, ESSDAI, clinESSDAI, ESSPRI, and treatment were compared. Longitudinal pediatric data were analysed descriptively, and factors associated with systemic treatment initiation were explored. The final cohorts included 102 cSjD and 140 adult patients. Children more frequently presented with recurrent parotid enlargement (44% vs. 13%, p < 0.0001), cutaneous involvement (25% vs. 8%, p < 0.0001), lymphadenopathy (24% vs. 9%, p = 0.001), arthritis (17.7% vs. 6.4%, p = 0.006), and fever (12% vs. 3%, p = 0.006), whereas sicca symptoms were significantly less common (xerostomia 45% vs. 94%; xerophthalmia 36% vs. 86%; both p < 0.0001). cSjD showed higher ESSDAI (median 5.5 vs. 1.0, p < 0.0001), higher serum IgG, but lower anti-SSA/anti-SSB positivity. During a 26.4-months median follow-up, 87.5% initiated systemic treatment. In exploratory analyses, higher baseline ESR was independently associated with a shorter time to systemic treatment initiation. Compared with adult-onset SjD, cSjD showed more frequent systemic inflammatory involvement and less prominent sicca manifestations. Pediatric-specific classification criteria, outcome measures, and prospective studies to optimise management and improve long-term outcomes are still crucial unmet needs.

Arthritis Research & Therapy
Università Cattolica del Sacro Cuore (IT), University of Udine (IT), University of Padua (IT), University of Milan (IT), University of Campania "Luigi Vanvitelli" (IT), University of Ferrara (IT), IRCCS Materno Infantile Burlo Garofolo (IT), Istituto Giannina Gaslini (IT), Agostino Gemelli University Polyclinic (IT), University of Chieti-Pescara (IT), Azienda Ospedaliera Citta' della Salute e della Scienza di Torino (IT), Ospedale Santa Chiara (IT), Azienda Sanitaria Unità Locale di Reggio Emilia (IT), Meyer Children's Hospital (IT), Santobono Children's Hospital (IT), Ospedale Microcitemico (IT), University of Campania "Luigi Vanvitelli" (IT), Azienda Socio Sanitaria Territoriale Lariana (IT), Ospedale Regina Margherita (IT), Ospedale Pediatrico Giovanni XXIII (IT), Ospedale Vito Fazzi (IT), Istituti di Ricovero e Cura a Carattere Scientifico (IT), ASST Fatebenefratelli Sacco (IT), Azienda sanitaria universitaria Friuli Centrale (IT), University of Naples Federico II (IT), University of Genoa (IT), Sapienza University of Rome (IT)
Good health and well-being
Openalex Percentile: Top 13%
Salivary Gland Disorders and Functions
DOIOpen Access PDFOpenAlex
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